[Usefulness of SPECT and MRI in the diagnosis of atypical parkinsonism]. / Diagnostisk nytte av SPECT- og MR-undersøkelse ved atypisk parkinsonisme.

BACKGROUND: Clinico-pathological studies have shown that only three out of four patients with parkinsonism have idiopathic Parkinson's disease. In patients with so-called Parkinson plus syndrome, the degeneration in the brain is more widespread and the variety of neurological signs greater than in Parkinson's disease. The differentiation of these syndromes from Parkinson's disease can be difficult. Single photon emission computed tomography (SPECT) and magnetic resonance imaging (MRI) can be of value in the differential diagnosis of parkinsonism. MATERIAL AND METHODS: We present three patients with atypical parkinsonism in whom MRI and SPECT with beta-CIT and epidepride was performed in addition to the clinical evaluation. RESULTS: The three patients all had a rapidly developing symmetric akinetic-rigid syndrome that responded poorly to levodopa. MRI showed findings regarded as typical for multiple system atrophy in two patients, but only nonspecific findings in the third patient. SPECT with beta-CIT showed a pronounced bilateral and relatively symmetric reduction in the striatal dopaminergic activity in all patients. SPECT with epidepride showed a clearly reduced striatal D2-receptor binding bilaterally in only one of the patients. INTERPRETATION: In patients with atypical parkinsonism, MRI and SPECT with beta-CIT and epidepride can give valuable support to the clinical diagnosis of a Parkinson plus syndrome.

[Guillain-Barre syndrome. Current treatment principles in the light of clinical aspects]. / Guillain-Barrés syndrom. Moderne behandlingsprinsipper belyst ved et klinisk materiale.

The Guillain-Barré's syndrome, or acute polyradiculoneuropathy, is a monophasic neurological disease affecting 50-100 persons a year in Norway. In addition to peripheral paresis, respiratory and autonomic disturbances may occur. We present 22 patients, mean age 34.8 years, including four children between four and six years of age, who all received plasma exchange treatment. All our patients reported symptoms of a modest infection average 19 days before the neurological symptoms appeared. All patients had walking difficulties, half of them were unable to walk without assistance. There were cranial nerve findings in 18 patients, and nine had autonomic disturbances when admitted. All except one had increased protein contents in the spinal fluid as well as pathological findings in electrophysiological investigations. They received on average 8.6 plasma exchanges. In spite of such treatment, the total mortality rate has not decreased substantially. Two of our patients died, and three developed severe permanent paresis. Further studies on pathogenesis will be required to increase treatment success.

Cyclosporin A and brain excitability studied in vitro.

PURPOSE: Seizures are frequently observed after organ transplantations. This has been attributed to a direct effect of cyclosporin A (CsA) on the brain, although other mechanisms may also be of importance. The aim of this study was to investigate possible acute and direct effects of CsA on neuronal excitability. METHODS: Female rat hippocampal slices were perfused with CsA solutions containing 400 (n = 4), 1,000 (n = 4), 2,000 (n = 6), 8,000 (n = 8) microg/L CsA or control (n = 8) for 30 min, or penicillin, 2,000 IE/ml (n = 7). Actual concentrations of CsA were measured in the perfusate drawn from the slice chamber. To study CsA accumulation in the slices, uptake of radioactive CsA was measured in 12 living and 11 dead slices. RESULTS: Despite a significant accumulation of CsA in the living neuronal slices, no effects were observed on prevolley, field excitatory postsynaptic potential (fEPSP), or population spike amplitude. Penicillin, however, led to epileptiform activity within 10 min in all cases. Concentrations of CsA in the perfusate from the slice chamber were about half the calculated levels, demonstrating that the slices had been exposed to actual CsA concentrations in the range of approximately 200-4,000 microg/L CsA. CONCLUSIONS: Our results demonstrate a lack of acute effects of CsA on neuronal excitability within clinically relevant concentrations despite an active accumulation of the drug in the slices. Long-term effects on brain tissue, indirect metabolic effects, or synergistic effects may be responsible for the neurotoxicity of the drug.

[Atrial septal aneurysm and cerebrovascular diseases]. / Atrieseptumaneurisme og cerebrovaskulaer sykdom.

Atrial septal aneurysm has a prevalence of 1% in the general population. A case is presented where a clinically healthy man had three cerebellar ischemic events on the basis of an atrial septal aneurysm. The literature on the subject is reviewed, and the importance of transoesophageal echocardiography is emphasized.

[Familial protein S deficiency]. / Familiaer protein S-mangel.

Protein S deficiency is an autosomal dominant inherited disease. The authors describe a family in which 27 of the 52 members had a deficiency of S protein. Eight family members have suffered from thromboembolic disease.